Leichter D A, Sullivan I, Gersony W M
Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, New York.
J Am Coll Cardiol. 1989 Nov 15;14(6):1539-44. doi: 10.1016/0735-1097(89)90395-1.
Discrete subvalvular aortic stenosis is a progressive lesion. Most reported cases have had a measurable left ventricular-aortic gradient that progressed in severity. This report describes 35 patients in whom no significant left ventricular-aortic obstruction was noted at initial cardiac catheterization, but who later were shown to have significant subvalvular aortic stenosis. In 24 of the 35 cases, absence of a significant left ventricular-aortic gradient (less than or equal to 10 mm Hg) was documented at initial cardiac catheterization. In 11 patients, a left ventricular-aortic pressure gradient was not obtained or not sought in the absence of clinical evidence of an obstructive lesion. In each case, discrete subaortic stenosis was not noted on angiography. Associated lesions included ventricular septal defect in 7, patent ductus arteriosus in 12, coarctation of the aorta in 8, pulmonary stenosis in 3, atrioventricular canal in 2 and miscellaneous lesions in 3 cases. The 35 patients had documentation of subvalvular aortic stenosis 3 months to 19 years after their initial study based on repeat catheterizations in 26, echocardiography in 6 or discovery at surgery in 3 cases. There were eight children with coarctation and no left ventricular-aortic gradient who developed significant subvalvular stenosis at a median of 2 years 9 months after initial cardiac catheterization. Of 30 patients with associated lesions, 23 had surgical intervention before development of subvalvular aortic stenosis, but only 5 of 17 patients with ventricular septal defect had surgical repair or palliation specifically for the interventricular communication before development of subvalvular aortic stenosis.(ABSTRACT TRUNCATED AT 250 WORDS)
离散性瓣下主动脉狭窄是一种进行性病变。大多数报道的病例都有可测量的左心室-主动脉压差，且严重程度不断进展。本报告描述了35例患者，这些患者在初次心导管检查时未发现明显的左心室-主动脉梗阻，但后来被证明患有明显的瓣下主动脉狭窄。在35例病例中的24例，初次心导管检查记录显示无明显的左心室-主动脉压差（小于或等于10 mmHg）。在11例患者中，由于缺乏梗阻性病变的临床证据，未获得或未寻求左心室-主动脉压力梯度。在每个病例中，血管造影均未发现离散性主动脉下狭窄。相关病变包括7例室间隔缺损、12例动脉导管未闭、8例主动脉缩窄、3例肺动脉狭窄、2例房室通道以及3例其他病变。根据26例患者的重复心导管检查、6例患者的超声心动图检查或3例患者手术时的发现，这35例患者在初次检查后3个月至19年有瓣下主动脉狭窄的记录。有8例合并主动脉缩窄且无左心室-主动脉压差的儿童在初次心导管检查后中位数2年9个月时出现了明显的瓣下狭窄。在30例有相关病变的患者中，23例在瓣下主动脉狭窄出现之前接受了手术干预，但在17例室间隔缺损患者中，只有5例在瓣下主动脉狭窄出现之前专门针对室间交通进行了手术修复或姑息治疗。（摘要截短于250字）
